McAdoo SP, et al. - In this single-centre cohort study, it was demonstrated that a combination of oral corticosteroids, rituximab and low-dose pulsed intravenous cyclophosphamide followed by maintenance with azathioprine and tapered steroid for the treatment of biopsy-proven renal involvement in anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) demonstrated potential superiority over current standards of care.

• Researchers performed a single-centre cohort study including 66 patients treated with a combination of oral corticosteroids, rituximab and low-dose pulsed intravenous cyclophosphamide followed by a maintenance regimen of azathioprine and tapered steroid for the treatment of biopsy-proven renal involvement in AAV.
• A median follow-up of 56 months was performed.
• Case–control analysis with 198 propensity-matched cases from European Vasculitis Study Group (EUVAS) trials compared long-term differences in relapse-free, renal and patient survival.

• Data showed that at entry, the median Birmingham Vasculitis Activity Score (BVAS) was 19 and estimated glomerular filtration rate was 25 mL/min.
• At 6 months, cumulative doses of rituximab, cyclophosphamide and corticosteroids were 2, 3 and 4.2 g, respectively.
• Disease remission was achieved by a total of 94% of patients by 6 months (BVAS < 0) and patient and renal survival were 84 and 95%, respectively, at 5 years.
• Findings demonstrated that ANCA-negative status was detected in a total of 84% and 57% remained B cell deplete at 2 years, which was associated with low rates of major relapse (15% at 5 years).
• During long-term follow-up, the serious infection rate was 1.24 per 10 patient-years.
• Notably, in comparison with propensity-matched patients enrolled in EUVAS trials, treatment with this regimen was found to be associated with an attenuated risk of death {hazard ratio [HR] 0.29 [95% confidence interval (CI) 0.125–0.675], P=0.004}, progression to end-stage renal disease (ESRD) [HR 0.20 (95% CI 0.06–0.65), P=0.007] and relapse [HR 0.49 (95% CI 0.25–0.97), P=0.04].